New Staged Repair of Neonatal Tetralogy of Fallot with Severe Absent Pulmonary Valve Syndrome
10.4326/jjcvs.44.97
- VernacularTitle:ファロー四徴症，肺動脈弁欠損に対する新生児期姑息術の工夫
- Author:
Hiroo Kinami
;
Kiyozo Morita
;
Yoshihiro Ko
;
Gen Shinohara
;
Kazuhiro Hashimoto
- Publication Type:Journal Article
- Keywords:
tetralogy of Fallot;
absent pulmonary valve syndrome;
neonatal;
staged repair
- From:Japanese Journal of Cardiovascular Surgery
2015;44(2):97-102
- CountryJapan
- Language:Japanese
-
Abstract:
Primary repair of the tetralogy of Fallot with absent pulmonary valve syndrome (TOF/APV) is associated with high mortality rates of 17-33%, especially in neonates. Our standard strategy involves a staged repair with a first palliation, performed during the neonatal period, that includes main pulmonary septation with an ePTFE patch, pulmonary arterioplasty for reduction of vascular dilation, and a modified Blalock-Taussig shunt. We performed successful repairs on two neonates with TOF/APV, one symptomatic and the other non-symptomatic, with this strategy. Case 1 : A 7-day-old boy had TOF/APV, with progressively worsening respiratory distress. His left bronchi, superior vena cava and left atrium were compressed by a dilated pulmonary artery, which was repaired by emergency surgery. Decreasing the diameter of the pulmonary artery (PA index from 2,550 to 525) relieved the compressed organs. Case 2 : A 16-day-old boy with TOF/APV with a main pulmonary artery that increased in diameter from 8 to 17 mm in the course of a single day. He was treated in the same fashion as Case 1. At 1 year of age, an intracardiac repair with tricuspid anuuloplasty was performed successfully. This strategy is much safer than a primary repair and is a good choice for neonatal repair of TOF/APV.
